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Antiphospholipid Patients Admitted in the Intensive Care Unit: What Must The Rheumatologist Know?

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Abstract

Purpose of the review

Antiphospholipid syndrome (APS) is a rare systemic autoimmune disorder that can escalate into a 'thrombotic storm' called the catastrophic antiphospholipid syndrome (CAPS), frequently requiring ICU admission for multiple organ failure. This review aims to offer insight and recent evidence on critically-ill APS patients.

Recent findings

The CAPS classification criteria define this condition as the involvement of at least three organs/systems/tissues within less than a week, caused by small vessel thrombosis, in patients with elevated antiphospholipid antibodies levels. These criteria do not encompass the full spectrum of critically-ill thrombotic APS patients and they need to be cautiously used for the bedside diagnosis of CAPS. Thrombocytopenia is the laboratory hallmark of CAPS, sometimes dropping below 20G/L, but a complete thrombotic microangiopathy pattern is infrequent. Anticoagulation is the pivotal treatment for APS and CAPS, associated with improved outcome. Triple therapy – the combination of anticoagulation, high-dose corticosteroids, and either plasma exchange or intravenous immunoglobulins – remains the standard treatment for CAPS patients. Eculizumab, an anti-C5 monoclonal antibody, may be useful in refractory patients.

Summary

Despite significant progress, CAPS mortality rate remains high. Its diagnosis and management are complex, requiring a close multidisciplinary cross talk between APS specialists and intensivists.

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Abbreviations

aβ2GP1:

Anti-β2-glycoprotein 1 autoantibodies

aCL:

Anti-cardiolipin autoantibodies

ADAMTS 13:

A disintegrin and metalloprotease with thrombospondin type I repeats-13

aPL:

Antiphospholipid autoantibodies

APS:

Antiphospholipid syndrome

CAPS:

Catastrophic antiphospholipid syndrome

CVD:

Cardiovascular disease

ICU:

Intensive care unit

INR:

International normalized ratio

LA:

Lupus anticoagulant

LMWH:

Low molecular weight heparin

LVEF:

Left ventricle ejection fraction

SLE:

Systemic lupus erythematosus

UFH:

Unfractioned heparin

VKA:

Vitamin K agonist

VTE:

Venous thromboembolism

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Authors and Affiliations

  1. Hôpital La Pitié–Salpêtrière, Service de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France

    Quentin Moyon, Alain Combes & Marc Pineton de Chambrun

  2. AP-HP, Hôpital La Pitié–Salpêtrière, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique, Syndrome Des Anticorps Anti-Phospholipides Et Autres Maladies Auto-Immunes Systémiques Rares, Sorbonne Université, Paris, France

    Quentin Moyon, Alexis Mathian, Matthias Papo, Zahir Amoura & Marc Pineton de Chambrun

  3. Inserm, Centre d’Immunologie Et Des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France

    Alexis Mathian, Zahir Amoura & Marc Pineton de Chambrun

  4. Institut de Cardiométabolisme Et Nutrition (ICAN), Sorbonne Université, INSERM, UMRS_1166-ICAN, Paris, France

    Alain Combes & Marc Pineton de Chambrun

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  1. Quentin Moyon
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Moyon, Q., Mathian, A., Papo, M. et al. Antiphospholipid Patients Admitted in the Intensive Care Unit: What Must The Rheumatologist Know?. Curr Rheumatol Rep (2024). https://doi.org/10.1007/s11926-024-01148-7

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